DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20173601

γδ T-cell lymphoma: a rare entity mimicking hemophagocytic syndrome-report of two cases

Ujwala Maheshwari, Kalyani Mahore, Evith Pereira, Reeta Dhar

Abstract


γδ T-cell lymphoma is a rare extranodal and systemic neoplasm which accounts for less than 1% of all NHL having peak incidence in adolescents and young adults with a male predominance. Study report two cases, 29 and 25 years male presented with fever, abdominal pain and pancytopenia; one had hepatosplenomegaly while other had marked splenomegaly respectively and both showing feature of hemophagocytosis. Both underwent splenectomy. On gross examination, the spleen was markedly enlarged and shows a deep brown cut-surface. Histologically the normal structure of the spleen is totally effaced by a monotonous lymphoid population consisting of medium-large sized lymphoid cells with vesicular chromatin and small eosinophilic nucleolus with tumor cells showing moderate amount of eosinophilic cytoplasm. Liver biopsy showed regenerative changes in parenchyma and sinusoidal involvement by tumour cells. Peripheral smear revealed atypical lymphocytes with pancytopenia. Bone marrow aspiration showed moderate neoplastic infiltrate by lymphoid cells. Immunophenotypic study showed tumour cells expressing CD3, CD56, CD 2 (dim), TIA-1, TCR-gamma. The tumour cells were immunonegative for CD20, CD5, CD4, CD8, granzyme B, CD7, CD10 and CD 23. Immunophenotypic study is must for diagnosis of   γδ T-cell lymphoma which can mimic hemophagocytic syndrome.


Keywords


Splenomegaly, γδ T-cell lymphoma

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References


Cooke CB, Krenacs L, Stetler-Stevenson M, Greiner TC, Raffeld M, Kingma DW, et al. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood. 1996;88:4265-74.

Bordessoule D, Gaulard P, Mason DY. Preferential localisation of human lymphocytes bearing gamma delta T cell receptors to the red pulp of the spleen. J Clin Pathol. 1990;43(6):461-4.

Ross CW, Schnitzer B, Sheldon S, Braun DK, Hanson CA. Gamma/delta T-cell post transplantation lymphoproliferative disorder primarily in the spleen. Am J Clin Pathol. 1994;102:310-5.

Salhany KE, Feldman M, Peritt D, Nowell PC. Cytotoxic T-lymphocyte differentiation and cytogenetic alterations in gamma delta hepatosplenic T-cell lymphoma and posttransplant lymphoproliferative disorders. Blood. 1997;89:3490-1.

Roelandt PR, Maertens J, Vandenberghe P, Verslype C, Roskams T, Aerts R, et al. Hepatosplenic gamma delta T-cell lymphoma after liver transplantation: report of the first 2 cases and review of the literature. Liver Transplant. 2009;15(7):686-92.

Rothenberg ME, Weber WE, Longtine JA, Hafler DA. Cytotoxic gamma delta I lymphocytes associated with an Epstein-Barr virus-induced post transplantation lymphoproliferative disorder. Clin Immunol Immunopathol. 1996;80(3):266-72.

Ozaki S, Ogasahara K, Kosaka M, Inoshita T, Wakatsuki S, Uehara H, et al. Hepatosplenic gamma delta T-cell lymphoma associated with hepatitis B virus infection. J Med Invest. 1998;44:215-7.

Gaulard P, Jaffe E, Krenacs L, Macon WR. Hepatosplenic T-cell lymphoma. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, eds. WHO classification of tumours of hematopoietic and lymphoid tissues. 4th ed. Lyon: IARC; 2008:292-293.

Farcet JP, Gaulard P, Marolleau JP, Le Couedic JP, Henni T, Gourdin MF, et al. Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta. Blood. 1990;75:2213-9.

Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M, et al. Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas. Am J Pathol. 1990;137(3):617-28.

De Wolf-Peeters C, Achten R. Gamma delta T-cell lymphomas: a homogeneous entity? Histopathol. 2000;36(4):294-305.

Allory Y, Challine D, Haioun C, Copie-Bergman C, Delfau-Larue MH, Boucher E, et al. Bone marrow involvement in lymphomas with hemophagocytic syndrome at presentation: a clinicopathologic study of 11 patients in a Western institution. Am J Surg Pathol. 2001;25(7):865-74.

Gaulard P, Belhadj K, Reyes F. Gamma delta T-cell lymphomas. Semin Hematol. 2003;40(3):233-43.

Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE, et al. Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. Ann Oncol. 2009;20(6):1080-5.

Swerdlow S, McColl K, Rong Y, Lam M, Gupta A, Distelhorst CW. Apoptosis inhibition by Bcl-2 gives way to autophagy in glucocorticoid-treated lymphocytes. Autophagy. 2008;4(5):612-20.

Weidmann E. Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990. Leukaemia. 2000;14(6):991-7.

Iannitto E, Tripodo C. How I diagnose and treat splenic lymphomas. Blood. 2011;117(9):2585-95.

Vega F, Medeiros LJ, Gaulard P. Hepatosplenic and other gamma delta T-cell lymphomas. Am J Clin Pathol. 2007;127(6):869-80.